RT Journal Article
T1 Langerhans cell histiocytosis. Advances in pathogenesis and clinical practice.
A1 Astigarraga, Itziar
A1 García-Obregón, Susana
A1 Pérez-Martínez, Antonio
A1 Gutiérrez-Carrasco, Ignacio
A1 Santa-María, Vicente
A1 Iturrate, Carmen Rodríguez-Vigil
A1 Reggiori, Mikael Lorite
A1 Carrillo, Thais Murciano
A1 Torrent, Montse
A1 Grupo de Histiocitosis de la Sociedad Española de Hematología y Oncología Pediátricas (SEHOP), 
K1 BRAF inhibitors
K1 Eosinophilic granuloma
K1 Granuloma eosinófilo
K1 Histiocitosis
K1 Histiocitosis de células de langerhans
K1 Histiocytic disorders
K1 Histiocytosis
K1 Inhibidores BRAF
K1 Inhibidores MEK
K1 Langerhans cell histiocytosis
K1 MEK inhibitors
K1 Medicina de precisión
K1 Precision medicine
K1 Síndromes histiocíticos
K1 Targeted therapy
K1 Terapia dirigida
AB Langerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical presentation and biological behaviour, so it may mimic different diseases. Performance of different clinical assessments and laboratory and imaging tests is recommended to determine the extent of involvement, which may be of a single location or multisystemic, and the presence or absence of dysfunction in risk organs, such as the haematopoietic system, liver and spleen. The diagnosis must be confirmed by histological examination of a biopsy sample. Molecular tests have identified mutations in the mitogen-activated protein kinase (MAPK) pathway, which has expanded treatment options. The diagnosis is complex and there is controversy regarding the management of certain cases. Treatment recommendations depend on the location of the lesions and the extent of involvement. International collaborative studies have demonstrated the effectiveness of prolonged combination therapies such as vinblastine and prednisone in severe or multisystemic forms, and anti-inflammatory drugs such as indomethacin and other cytostatic combinations have proven beneficial. Langerhans cell histiocytosis is a good example of the importance of precision medicine and the benefit of identifying molecular targets, common to different neoplasms, to develop new therapies. MAPK pathway inhibitors offer an alternative treatment option in refractory cases and neurodegenerative forms of LCH. Molecular testing can contribute to the prognosis, treatment and follow-up of LCH, especially in severe forms of disease.
YR 2022
FD 2022-07-20
LK http://hdl.handle.net/10668/21924
UL http://hdl.handle.net/10668/21924
LA en
DS RISalud
RD Apr 19, 2025